Tayama M, Hashimoto T, Mori K, Miyazaki M, Yoshimoto T, Kuroda Y
Department of Pediatrics, University of Tokushima School of Medicine.
No To Hattatsu. 1994 Jul;26(4):308-12.
Neurophysiologic studies on six patients (five lissencephaly and one schizencephaly) with migration disorder were performed in order to evaluate the CNS function by multimodality evoked potentials. All cases revealed abnormalities in SSEP, four cases lost components below P 3 or had low voltage of P 3 indicating brainstem dysfunction in the somatosensory pathway. Two cases lost component below N 1 indicating sensory cortical dysfunction. Three cases revealed ABR abnormalities including a low amplitude of wave V, and one case revealed a prolonged peak latency of wave I and a low amplitude of all components. Two cases revealed abnormalities of poor cortical components in SEP, two cases in VEP, and three cases in MLR. Five cases had normal findings in P-SEP and three cases in VEP. The result of this study demonstrates that patients with migration disorder are frequently associated with cortical, brainstem and peripheral dysfunctions and has heterogeneity in pathophysiology.
对6例患有神经元移行障碍的患者(5例无脑回畸形和1例脑裂畸形)进行了神经生理学研究,以通过多模态诱发电位评估中枢神经系统功能。所有病例的体感诱发电位(SSEP)均显示异常,4例在P3波以下成分消失或P3波电压降低,提示体感通路脑干功能障碍。2例在N1波以下成分消失,提示感觉皮层功能障碍。3例听觉脑干诱发电位(ABR)异常,包括V波振幅降低,1例I波波峰潜伏期延长且所有成分振幅降低。2例体感诱发电位(SEP)皮层成分不良、2例视觉诱发电位(VEP)和3例运动诱发电位(MLR)异常。5例P-SEP结果正常,3例VEP结果正常。本研究结果表明,患有神经元移行障碍的患者常伴有皮层、脑干和外周功能障碍,且在病理生理学上具有异质性。